IBA57抗体;IBA57 Antibody—艾普蒂

+ +

•  

   

     The image is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using P12990(IBA57 Antibody) at dilution 1/65. (Original magnification: ×200)    

  
  

•  

   

     The image is immunohistochemistry of paraffin-embedded Human brain tissue using P12990(IBA57 Antibody) at dilution 1/65. (Original magnification: ×200)    

  
  

The IBA57 antibody is a crucial tool for studying the mitochondrial iron-sulfur cluster (Fe-S) assembly system. IBA57 is a mitochondrial protein involved in the biosynthesis of Fe-S clusters, essential cofactors for enzymes in critical cellular processes like electron transport, DNA repair, and metabolic regulation. It functions as part of the mitochondrial ISC machinery, working alongside other proteins (e.g., ISCU, NFU1) to assemble and transfer Fe-S clusters to target apoproteins. Mutations in the IBA57 gene are linked to mitochondrial disorders, such as multiple mitochondrial dysfunctions syndrome type 3 (MMDS3), characterized by severe neurological impairment and lactic acidosis.

Antibodies against IBA57 enable researchers to investigate its expression, localization, and interactions via techniques like Western blotting, immunofluorescence, and immunoprecipitation. These tools are vital for elucidating its role in Fe-S biogenesis and disease mechanisms. Studies using IBA57 antibodies have revealed reduced protein levels in patient-derived cells with pathogenic variants, confirming its clinical relevance. Additionally, they help explore IBA57's potential involvement in cancer, where altered Fe-S metabolism may drive tumor progression. Most IBA57 antibodies are raised in rabbits or mice using recombinant protein fragments, and their specificity is validated through knockdown/knockout controls. Overall, IBA57 antibodies are indispensable for advancing understanding of mitochondrial biology and associated pathologies.